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Peripheral primitive neuroectodermal tumour of left ventricular wall origin: a rare case
Ying Chai1, Lijian Huang, Lan Yue
1Department of Thoracic Surgery, 2nd Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
Acta Cardiologica
|November 7, 2007
Summary
A rare peripheral primitive neuroectodermal tumour (pPNET) of the left ventricle was resected. Cardiac transplantation was considered due to tumor recurrence without distant metastases.
Area of Science:
- Cardiology
- Oncology
- Pathology
Background:
- Primary cardiac tumors are rare, with primitive neuroectodermal tumors (PNETs) being exceptionally uncommon in the myocardium.
- This case presents a peripheral primitive neuroectodermal tumor (pPNET) arising from the left ventricular wall.
Observation:
- The tumor exhibited characteristic microscopic features of pPNET, including small, round, deeply basophilic nuclei with dense chromatin.
- The tumor demonstrated growth towards the pericardial cavity, a pattern not previously reported for myocardial PNETs.
Findings:
- A patient underwent resection of a left ventricular pPNET near the atrioventricular junction.
- Recurrence in situ was observed, with no evidence of distant metastases.
Implications:
- This case highlights the rarity of primary myocardial pPNET and the lack of established optimal treatment protocols.
- Orthotopic cardiac transplantation was considered as a treatment option for the recurrent tumor.
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